MANAGEMENT OF REFRACTORY LYMPHATIC LEAK
CASE 2: A newborn male is admitted to the neonatal ICU
for respiratory distress secondary to large bilateral pulmonary
effusions. The infant is intubated and multiple chest tubes are placed
to maintain adequate ventilation. Aspirated fluid is milky white in
color and confirmed chylous with a high triglyceride and lymphocyte
content. Peripheral laboratory evaluation is notable for
hypoalbuminemia, hypogammaglobulinemia and lymphocytopenia. In addition
to continuous chest tube drainage, acute interventions include complete
gut rest with total parenteral nutrition and intravenous lipids, IV
albumin replacement, and initiation of octreotide infusion. Despite
maximizing these interventions, chylous output remains >
20ml/kg/day. Intranodal Dynamic Contrast-Enhanced MR lymphangiography
(IN-DCMRL) demonstrates a diffuse Central Conducting Lymphatic Anomaly
(CCLA) with extensive abdominal and thoracic lymphangiomatosis.
Interventional radiology acutely performs lymphatic interstitial
embolization while hematology/oncology initiates oral sirolimus
pharmacotherapy. After 8 weeks of sirolimus therapy, although now
extubated and protein replete, the infant remains with a single chest
tube in place and continues to struggle advancing beyond a medium chain
triglyceride formula.
Surgical and endolymphatic strategies for treatment of congenital
chylous effusions and ascites are usually employed after failure of
medical therapies. The decision to perform an intervention depends on
factors related to local expertise. Many times, these invasive
procedures are performed in tandem or after prior procedures have failed
to stop chylous leakage.
Symptoms of refractory disease include unresponsive daily loss of chyle
exceeding 100 ml/day for a 5-day period, or symptomatic nutritional,
protein and/or electrolyte complications. Infants should not be allowed
to progress to a state of severe hepatic cholestasis without an attempt
at intervention.[21] Surgical and endolymphatic interventions
include drainage, shunting, pleurodesis, ligation of lymphatics,
lymphovenous anastomosis, and thoracic duct embolization.[22]